Figure 2

(A) Selected axial slices of brain MRI (T1 with gadolinium) performed two days after the last documented usual seizure, showing left parieto-occipital contrast enhancement in the leptominges and dilatation of veins of drainage. (B) Selected axial slices of 99mTc HMPAO SPECT performed seven days after the last documented crisis but during prolonged right-sided hemiparesis. The area of hyperperfusion involved the whole left parieto-occipital region (arrows). (C) After 29 days of aggressive antiepileptic therapy, the regions became hypoperfused. (D) Subtraction image between the two SPECT series, superposed on the MRI slices, showing an increased perfusion in the left parieto-occipital area. To perform subtraction, the two SPECT series were first normalized for injected activity and then spatially coregistered to the MRI using SPM (Wellcome Trust Center, London, UK).

Sturge-Weber syndrome is a sporadic congenital neurocutaneous disorder often related to varying degrees of motor impairment. The phenomenon of prolonged ictal paresis is a rare seizure sign that can be due to lesions affecting the centro-parietal lobe. Focal inhibitory motor seizures can be difficult to differentiate from other clinical entities such as stroke, migraine or postictal paresis. We describe the case of a 40-year-old patient suffering from Sturge-Weber syndrome, admitted due to prolonged right-sided hemiparesis following a usual seizure. Repeated EEGs during the prolonged deficit showed only intermittent left fronto-parietal sharp waves. 99mTc HMPAO-brain SPECT performed seven days after the last seizure showed a vast area of parieto-occipital hyperperfusion in the left hemisphere. Aggressive antiepileptic therapy dramatically improved the clinical symptoms and scintigraphic images, which corroborated the diagnosis of ictal paresis. This case highlights the role of SPECT in the evaluation of Sturge-Weber syndrome, not only to investigate progressive neurological deterioration, but also exacerbation of seizures or prolonged neurological deficits. In fact, it may be possible to document ongoing epileptic activity using SPECT, despite a non-contributory EEG, which may be of help in adapting a therapeutic strategy.